Neuroradiological Findings in the Spinocerebellar Ataxias

Meira, Alex Tiburtino; Arruda, Walter Oleschko; Ono, Sergio Eiji; de Carvalho Neto, Arnolfo; Raskin, Salmo; Camargo, Carlos Henrique F.; Teive, Hélio Afonso G.

Background: The spinocerebellar ataxias (SCAs) are a group of autosomal dominant degenerative diseases characterized by cerebellar ataxia. Classified according to gene discovery, specific features of the SCAs – clinical, laboratorial, and neuroradiological (NR) – can facilitate establishing the diagnosis. The purpose of this study was to review the particular NR abnormalities in the main SCAs.

Methods: We conducted a literature search on this topic.

Results: The main NR characteristics of brain imaging (magnetic resonance imaging or computerized tomography) in SCAs were: (1) pure cerebellar atrophy; (2) cerebellar atrophy with other findings (e.g., pontine, olivopontocerebellar, spinal, cortical, or subcortical atrophy; “hot cross bun sign”, and demyelinating lesions); (3) selective cerebellar atrophy; (4) no cerebellar atrophy.

Discussion: The main NR abnormalities in the commonest SCAs, are not pathognomonic of any specific genotype, but can be helpful in limiting the diagnostic options. We are progressing to a better understanding of the SCAs, not only genetically, but also pathologically; NR is helpful in the challenge of diagnosing the specific genotype of SCA.


  • thumnail for 682-Article Text-16239-1-10-20190926.pdf 682-Article Text-16239-1-10-20190926.pdf application/pdf 705 KB Download File

Also Published In

Tremor and Other Hyperkinetic Movements

More About This Work

Academic Units
Center for Parkinson's Disease and Other Movement Disorders
Published Here
December 17, 2019